title: Endolymphatic sac tumour (ELST). Case report of a rare tumour of the temporal bone, presenting as a mass in a cerebello-pontine angle.
abstract: Endolymphatic sac tumour (ELST) is a primary low-grade, locally invasive adenocarcinoma of the endolymphatic sac, characterized by the proliferation of cuboidal cells forming a papillotubular pattern and colloid-filled cysts. Rare in the general population, it coincides significantly with the presence of von Hippel-Lindau disease. The natural history, mechanisms underlying the early symptoms, anatomical origin of ELST and optimal timing of their treatment are unknown. In this study, we report a Polish male patient with sporadic ELST (without a family history of VHL disease) along with a review of literature. The light microscopic and immunohistochemical features as well as clinical presentation were typical of ELST.
authors.affiliation: Department of Pathomorphology, Jagiellonian University Medical College, Cracow, Poland.
authors.forename: Jacek
authors.initials: J
authors.lastname: Czopek
authors.forename: Marcin
authors.initials: M
authors.lastname: Waśko
authors.forename: Darius
authors.initials: D
authors.lastname: Adamek
mesh_terms: D000368:Aged; D001859:Bone Neoplasms / Q000175:diagnosis* / Q000601:surgery; D002530:Cerebellopontine Angle / Q000473:pathology* / Q000601:surgery; D003937:Diagnosis, Differential; D004712:Endolymphatic Sac / Q000473:pathology* / Q000601:surgery; D006801:Humans; D008297:Male; D009364:Neoplasm Recurrence, Local / Q000175:diagnosis* / Q000601:surgery; D013701:Temporal Bone / Q000473:pathology* / Q000601:surgery